Analysis of tear film in cystinosis patients treated with topical viscous cysteamine hydrochloride (Cystadrop(R))

摘要

Purpose The aim of this study was to evaluate in vivo the tear film in infantile nephropathic cystinosis patients with corneal crystals treated with topical viscous cysteamine hydrochloride (Cystadrop (R)). Methods Ten eyes of five patients with nephropathic cystinosis aged from 10 to 35 years were included in this study. The patients were under treatment with viscous cysteamine hydrochloride formulation containing 3.8 mg/mL cysteamine (vCH 0.55, equivalent to 0.55 CH; Cystadrop (R); Recordati rare Diseases, Puteaux, France) to reduce corneal crystal density. Five age and sex matched individuals were randomly selected as control group. Tear osmolarity testing (TearLab (TM)) was performed to assess the in vivo osmolarity of patients under treatment and compared to control group values. Tear film break-up time (TBUT) and basic tear secretion (Schirmer test) were also assessed. Results Mean tear osmolarity was 294.8 mOsms/L (+/- 10.4), with a mean absolute difference of 1.85 mOsms/L (+/- 2.13) between the eyes. There was no statistically significant difference between the osmolarity readings of cystinosis and the control group (294.8 +/- 10.4 vs 299.4 +/- 6.2mOsm/L, respectively; p = 0.39). The mean TBUT was 10.2 +/- 0.83 s in the study group versus 10 +/- 0.7 s in controls (p = 0.62). The mean Schirmer test score was 9.2 +/- 0.83 mm in the patients versus 10.2 +/- 0.83 mm in the controls (p = 0.14). Conclusions The TearLab (TM) osmolarity system test showed good reliability and precision in repeated measurements. This is the first report using the TearLab osmolarity system to assess tear film in patients with cystinosis treated with vCH 0.55. TearLab (TM) examination showed that the use of vCH 0.55 drops does not determine alterations of the tear film quality.